Learn / Metabolism

Metabolic pathways

Every FMK pathway as a clickable map. Pick one pathway to hammer it — next step, enzyme, regulator, cofactor, and the facts that get tested (NADPH and who needs it, rate-limiting steps, deficiencies) — or set it to all pathways for a mixed drill. Every answer says which enzyme and which pathway.

Pathway
Cholesterol synthesis · Cytosol / smooth ER — mainly liver. Enzymes sit beside the arrows; a ★ marks the rate-limiting step. The map scrolls inside its panel.
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Acetyl-CoA (cytosol)Acetyl-CoA (cytosol)Acetoacetyl-CoAAcetoacetyl-CoAHMG-CoA (cytosolic)HMG-CoA (cytosolic)MevalonateMevalonateIsoprenoid units (IPP)Isoprenoid units (IPP)Squalene (C30)Squalene (C30)LanosterolLanosterolCholesterol (C27)Cholesterol (C27)Bile acidsBile acidsVitamin DVitamin DSteroid hormonesSteroid hormonesThiolaseHMG-CoA synthase (cytosolic)HMG-CoA reductase ★Mevalonate kinases / decarboxylasePrenyl transferases → squalenesynthaseSqualene epoxidase → cyclase~19 steps (loses 3 methyl groups)Cholesterol 7α-hydroxylase(CYP7A1, rate-limiting)7-Dehydrocholesterol → UV-B (skin)→ 25-OH (liver) → 1,25-(OH)₂(kidney, 1α-hydroxylase)Desmolase / CYP450 side-chaincleavage (adrenal, gonads)

Cholesterol synthesis

Acetyl-CoA → acetoacetyl-CoA → HMG-CoA → mevalonate (HMG-CoA reductase, rate-limiting, 2 NADPH, statin target) → isoprenoids → squalene → lanosterol → cholesterol. '5 steps, 1 matters most.' Regulated short-term by AMPK/insulin/glucagon phosphorylation and long-term by SREBP-2/SCAP/INSIG.

Click any metabolite on the map for its reactions. Source: FMK-05.1 Cholesterol metabolism.

Reactions

StepEnzymeCofactorsRegulation
Acetyl-CoA (cytosol) → Acetoacetyl-CoA Thiolase
Acetoacetyl-CoA → HMG-CoA (cytosolic) HMG-CoA synthase (cytosolic) + acetyl-CoA
HMG-CoA (cytosolic) → Mevalonate HMG-CoA reductaseStatins (HMG-CoA reductase inhibition) → 2 NADPH → 2 NADP⁺
+ Insulin; Thyroid hormone; Low sterol → SREBP-2/SCAP to Golgi → ↑ transcription of reductase and LDL receptor
Statins (competitive); AMPK phosphorylation (low energy); Glucagon, epinephrine, glucocorticoids; High sterol → INSIG retains SREBP-2 in ER and tags reductase for degradation
Mevalonate → Isoprenoid units (IPP) Mevalonate kinases / decarboxylase 3 ATP
Isoprenoid units (IPP) → Squalene (C30) Prenyl transferases → squalene synthase
Squalene (C30) → Lanosterol Squalene epoxidase → cyclase NADPH, O₂
Lanosterol → Cholesterol (C27) ~19 steps (loses 3 methyl groups) NADPH
Cholesterol (C27) → Bile acids Cholesterol 7α-hydroxylase (CYP7A1, rate-limiting)Gallstones (cholesterol cholelithiasis) → NADPH, O₂
Cholesterol (C27) → Vitamin D 7-Dehydrocholesterol → UV-B (skin) → 25-OH (liver) → 1,25-(OH)₂ (kidney, 1α-hydroxylase)Vitamin D deficiency: rickets (child) / osteomalacia (adult) →
Cholesterol (C27) → Steroid hormones Desmolase / CYP450 side-chain cleavage (adrenal, gonads)Congenital adrenal hyperplasia (21α-hydroxylase deficiency) → NADPH

Conditions that live on this map

Congenital adrenal hyperplasia (21α-hydroxylase deficiency)Familial hypercholesterolemia (FH)Gallstones (cholesterol cholelithiasis)PCSK9 inhibitors (evolocumab, alirocumab)Statins (HMG-CoA reductase inhibition)Vitamin D deficiency: rickets (child) / osteomalacia (adult)

Blue pills sit on one specific arrow; grey ones are whole-pathway problems. Each opens the full condition card, which links back here with the arrow lit.

Facts worth knowing

Rate-limiting step

  • Rate-limiting enzyme of bile acid synthesis — Cholesterol 7α-hydroxylase (CYP7A1)
  • Rate-limiting enzyme of cholesterol synthesis — HMG-CoA reductase (The PFK-1 of cholesterol synthesis. Statins lower intracellular cholesterol → SREBP-2 → more LDL receptors — that is the actual LDL-lowering mechanism.)

Regulation

  • How statins actually lower plasma LDL — Less intracellular cholesterol → SREBP-2 activation → more LDL receptors on hepatocytes → more LDL cleared
  • Two independent brakes when sterol is high — INSIG holds SREBP-2/SCAP in the ER (no transcription) and marks existing HMG-CoA reductase for degradation

NADPH use

  • NADPH needed for cholesterol synthesis — 2 per mevalonate (HMG-CoA reductase) plus the squalene epoxidase and demethylation steps

Cofactor

  • Same intermediate, two fates: HMG-CoA — Cytosolic HMG-CoA reductase → mevalonate → cholesterol; mitochondrial HMG-CoA lyase → acetoacetate (ketone bodies)
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